Article ; Online: Hemophagocytic lymphohistiocytosis in adults.
2018 Volume 60, Issue 1, Page(s) 19–28
Abstract: Hemophagocytic lymphohistiocytosis (HLH), a rare but life-threatening condition characterized by uncontrolled inflammation, is increasingly recognized in adults. The management of adult onset HLH is challenging, in part due to gaps in current state of ... ...
Abstract | Hemophagocytic lymphohistiocytosis (HLH), a rare but life-threatening condition characterized by uncontrolled inflammation, is increasingly recognized in adults. The management of adult onset HLH is challenging, in part due to gaps in current state of knowledge on etiology, clinical presentation, diagnosis, and management. HLH secondary to triggers such as infections, autoimmune disorders, and malignancy are more commonly seen in adults although cases of familial form have also been reported. Underlying conditions such as sepsis, or malignancy could pose as major confounders while applying universal diagnostic criteria, and therefore could lead to delay in diagnosis. Despite advent of newer therapeutic agents, outcomes of adults continue to remain poor. Future efforts need to be orchestrated to develop evidence-based tailored therapies to improve outcomes of this under recognized heterogeneous entity. |
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MeSH term(s) | Adult ; Age of Onset ; Anti-Infective Agents/therapeutic use ; Clinical Trials as Topic ; Disease-Free Survival ; Drug Therapy, Combination ; Evidence-Based Medicine/methods ; Hematopoietic Stem Cell Transplantation/methods ; Humans ; Immunosuppressive Agents/therapeutic use ; Lymphohistiocytosis, Hemophagocytic/diagnosis ; Lymphohistiocytosis, Hemophagocytic/mortality ; Lymphohistiocytosis, Hemophagocytic/therapy ; Myeloablative Agonists/administration & dosage ; Neoplasm Recurrence, Local/mortality ; Neoplasm Recurrence, Local/therapy ; Prognosis ; Remission Induction/methods ; Salvage Therapy/methods ; Transplantation Conditioning/methods ; Transplantation, Homologous/methods | |||||
Chemical Substances | Anti-Infective Agents ; Immunosuppressive Agents ; Myeloablative Agonists | |||||
Language | English | |||||
Publishing date | 2018-07-03 | |||||
Publishing country | United States | |||||
Document type | Journal Article ; Review | |||||
ZDB-ID | 1042374-6 | |||||
ISSN | 1029-2403 ; 1042-8194 | |||||
ISSN (online) | 1029-2403 | |||||
ISSN | 1042-8194 | |||||
DOI | 10.1080/10428194.2018.1482543 | |||||
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Database | MEDical Literature Analysis and Retrieval System OnLINE |
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